BBSome是一个由8个保守蛋白组成的复合物，在纤毛运输中起作用(Nachury等，2007;Loktev等人，2008;Nachury et al, 2010;萧等，2012)。BBS基因突变导致Bardet-Biedl综合征，这是一种异质性纤毛病，在某些病例中以肥胖、失明、囊性肾病、视网膜色素变性、多指、智力发育迟缓和肾功能衰竭为特征(Tobin和Beales, 2009)。BBSome是ARL6/BBS3的主要效应体，ARL6/BBS3是一种小型的GTPase，它将BBSome和相关的膜蛋白招募到纤毛到膜上(Jin et al .， 2010;Nachury等人，2007;Zhang et al, 2011;Seo等人2011年)。BBSome还与RAB8A鸟嘌呤核苷酸交换因子RAB3IP相互作用，并以这种方式促进RAB8A被纤毛吸收(Nachury et al .， 2007)。 Components of the BBSome are enriched in beta propeller and TPR domains and have been shown to form linear arrays on liposomes (Jin et al, 2010). Where these arrays form, and how they contribute to ciliary targeting remains to be elucidated (Jin et al, 2010; reviewed in Nachury et al, 2010).In mammalian cells, formation of the BBSome depends on a BBS/CCT complex that consists of MKKS/BBS6, BBS10, BBS12 and 6 members of the CCT/TRiC family of chaperonins. The BBS/CCT complex interacts with a subset of the BBSome protein and plays a role in the BBS7 stability, promoting the formation of an intermediate "BBSome core complex" (Seo et al, 2010; Jin et al, 2010; Zhang el al, 2012).